Bu'Lock F A, Sood M, De Giovanni J V, Green S H
Department of Cardiology, Birmingham Children's Hospital, UK.
Arch Dis Child. 1999 Mar;80(3):267-70. doi: 10.1136/adc.80.3.267.
Examination of left ventricular function and conduction abnormalities in myotonic dystrophy.
Twelve patients (median age, 13.7 years) with myotonic dystrophy had detailed electrocardiography and echocardiography performed. Echocardiographic parameters were compared with body surface area (BSA) matched median normal values.
Fractional shortening was slightly reduced (by 28-29%) in three patients and three patients had mild mitral valve prolapse. Diastolic function was abnormal; isovolumic relaxation time (IVRT) and duration of early filling were prolonged compared with control values (median IVRT, 74 v 61 ms). Peak E velocity was increased (median, 0.82 v 0.78 m/s) but atrial phase filling was normal. Heart rate was reduced (median, 68 v 81 beats/min). Conduction abnormalities were common but showed no clear relations with diastolic abnormalities.
Young patients with myotonic dystrophy have myocardial diastolic dysfunction as well as abnormal electrophysiology. The prognostic implications of such abnormalities require further study.
研究强直性肌营养不良患者的左心室功能及传导异常情况。
对12例（中位年龄13.7岁）强直性肌营养不良患者进行了详细的心电图及超声心动图检查。将超声心动图参数与体表面积（BSA）匹配的正常中位值进行比较。
3例患者的缩短分数略有降低（降低28% - 29%），3例患者有轻度二尖瓣脱垂。舒张功能异常；与对照值相比，等容舒张时间（IVRT）和早期充盈持续时间延长（中位IVRT，74对61毫秒）。E峰速度增加（中位值，0.82对0.78米/秒），但心房期充盈正常。心率降低（中位值，68对81次/分钟）。传导异常常见，但与舒张异常无明显关系。
年轻的强直性肌营养不良患者存在心肌舒张功能障碍以及电生理异常。这些异常的预后意义需要进一步研究。
